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Related Experiment Videos

Brain stem MRI signal abnormalities in CADASIL

H Chabriat1, R Mrissa, C Levy

  • 1Service de Neurologie, Hôpital Lariboisière, the Service de Radiologie, Hôpital Saint-Antoine, Service de Neuroradiologie, Paris, France.

Stroke
|February 5, 1999
PubMed
Summary

Brain stem lesions are common in Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL), affecting 45% of patients. These abnormalities occur in areas supplied by perforating arteries, highlighting vascular architecture

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Area of Science:

  • Neurology
  • Neuroimaging
  • Vascular Neurology

Background:

  • Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) is an arteriopathy caused by Notch 3 gene mutations.
  • MRI signal abnormalities in CADASIL increase with age and show regional vulnerability in hemispheric subcortical areas.
  • The distribution of brain stem lesions in CADASIL has not been previously reported.

Purpose of the Study:

  • To investigate the prevalence, distribution, and clinical significance of brain stem signal abnormalities in CADASIL patients.
  • To correlate brain stem lesion patterns with the known angioarchitecture of cerebral vasculature in CADASIL.

Main Methods:

  • Retrospective review of MRI scans from 68 patients with CADASIL and hemispheric white matter abnormalities.

Related Experiment Videos

  • Assessment of T2-weighted and T1-weighted images for signal abnormalities in the brain stem.
  • Analysis of lesion location (pons, mesencephalon, medulla) and characteristics.

    • Main Results:

    • Brain stem signal abnormalities were detected in 45% of the studied CADASIL patients.
    • The pons was the most frequently affected brain stem region (100%), followed by the mesencephalon (69%) and medulla (35%).
    • T1-weighted hypointensities were observed in two-thirds of affected subjects; superficial brain stem lesions and cerebellar lesions were absent.

    Conclusions:

    • Brain stem signal abnormalities in CADASIL predominantly occur in territories supplied by perforating arteries.
    • These findings align with hemispheric lesion patterns, emphasizing the role of vascular angioarchitecture in CADASIL manifestation.
    • The study underscores that CADASIL, a systemic vessel wall disease, presents primarily as a neurological condition due to specific vascular vulnerabilities.